FAQ, part 1

What is HCM?

Hypertrophic cardiomyopathy. "Hypertrophic" simply means extra overgrowth of tissue. "Cardiomyopathy" means a disease of the heart muscle itself (not necessarily of the arteries, which is more common and is generally the first thing one thinks of upon hearing 'heart disease.')

What's wrong with your heart specifically?

To get at this, it's helpful to thoroughly understand the anatomy of a healthy heart. Click on this diagram, and let's clarify a few items. See the big pink areas in the center? They're the heart's chambers - they fill and unfill with blood. The beige line running from the center downward to the right is the septum, which is the muscle tissue that separates the left ventricle from the right ventricle. The beige ring around the outside of the entire heart is the muscular wall. So beige = muscle, pink = chamber.

Now, imagine that the top portion of the septum is about twice as wide as it should be, extending to the right, almost all the way to the mitral valve. That's my heart, and with every beat, the mitral valve comes into contact with the septum (they shouldn't be anywhere near each other). In a normal heart, the blood flows upward out of the left ventricle. In my heart, the blood has nowhere to go - it's obstructed by the contact between the mitral valve and the septum. So pressure builds up in the left ventricle, as well as further back in the system - it's like a garden hose that's kinked. That pressure build-up (called the gradient) varies continuously - that's why some days are better than others.

What causes the pressure to vary?

Dehydration, fatigue, eating (digestion), heat (especially with high humidity) and exercise all make the gradient worse.

What happens then?

In highly scientific terms: I feel really crummy. It becomes harder to breathe (and my breathing rate goes up), there's a sensation of chest pressure, and I may feel lightheaded or dizzy.

What can the doctors do about it?

First, medications to try and reduce the gradient. I'm on a bunch at some high doses. Second, open-heart surgery to remove the extra muscle in the septum. That's where I'm headed.

Can't they put in a stent?

Stents are for arteries that have become obstructed. If you go back to the diagram, you'll see that the problem isn't with the arteries (which lie on the surface of the heart), but rather inside the heart itself. Surgery is the only way to get there (well, except for septal alcohol ablation, which is another topic).

More Q&A soon.

Take care,

Michael